Consensus Statement and Guidelines on the Management of Paragangliomas of the Head and Neck

ABSTRACT

Paragangliomas (PGLs) of the head and neck (H&N) are uncommon tumors that either arise spontaneously or as part of an inherited syndrome. Hereditary PGL is an autosomal-dominant tumor predisposition syndrome in which an affected individual has greatly increased risk of developing PGL at any or several sites in the autonomic nervous system. The mode of inheritance for some is affected by maternal imprinting. These tumors are generally very slow growing, often taking a decade to double in size. A few are or can become malignant and can metastasize widely. Because of their site of origin, patients with these tumors may develop cranial nerve deficits that have a significant impact on their quality of life. Patients may present to specialists from widely differing disciplines, and some of these may not appreciate the full implications of their patient’s disease. As a result, management can become fragmented or inappropriate, and some aspects of care may even be overlooked. This article is the distillation of consensus opinion derived from current published and unpublished data in this field, with particular reference to the management of temporal bone PGLs. We propose guidelines for the management of both sporadic and hereditary PGLs. A multidisciplinary team approach to the management of this complex disorder is advocated. Progress could be made by adopting these guidelines and by widespread dissemination of standardized information. Collaborative research should be promoted with the aim of harnessing advances in molecular genetics to develop targeted therapies for patients, particularly those with hereditary PGL.

KEYWORDS

Head and neck paraganglioma - management - surgery - genetics - molecular biology

REFERENCES

• 1 Neumann H PH, Pawlu C, Peçzkowska M et al.. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations.  JAMA. 2004;  292(8) 943-951
• 2 Boedeker C C, Neumann H PH, Offergeld C et al.. Clinical features of paraganglioma syndromes.  Skull Base. 2009;  19 17-25
• 3 Jansen J C, van der Berg R, Kuiper A, van der May A G, Zwinderman A H, Cornelisse C J. Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal.  Cancer. 2000;  88 2811-2816
• 4 Gimm O, Armanios M, Dziema H, Neumann H P, Eng C. Somatic and occult germ-line mutations in SDHD, a mitochondrial complex II gene, in nonfamilial pheochromocytomas.  Cancer Res. 2000;  60 6822-6825
• 5 Gimenez-Roqueplo A P, Favier J, Rustin P et al.. Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas.  Cancer Res. 2003;  63 5615-5621
• 6 Baysal B E. Hereditary paraganglioma targets diverse paraganglia.  J Med Genet. 2002;  39 617-622
• 7 Niemann S, Müller U. Mutations in SDHC cause autosomal dominant paraganglioma, type 3.  Nat Genet. 2000;  26 268-270
• 8 Astuti D, Latif F, Dallol A et al.. Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma.  Am J Hum Genet. 2001;  69 49-54
• 9 Birch-Machin M A, Taylor R W, Cochran B, Ackrell B A, Turnbull D M. Late-onset optic atrophy, ataxia, and myopathy associated with a mutation of a complex II gene.  Ann Neurol. 2000;  48 330-335
• 10 Hensen E F, Jordanova E S, van Minderhout I J et al.. Somatic loss of maternal chromosome 11 causes parent-of-origin-dependent inheritance in SDHD-linked paraganglioma and phaeochromocytoma families.  Oncogene. 2004;  23 4076-4083
• 11 Neumann H PH, Pawlu C, Peçzkowska M et al.. Distinct clinical features characterize paraganglioma syndromes associated with SDHB and SDHD gene mutations.  JAMA. 2004;  292 943-951
• 12 Vogl T J, Bisdas S. Differential diagnosis of jugular foramen lesions.  Skull Base. 2009;  19 3-16
• 13 Fisch U, Mattox D. Microsurgery of the Skull Base. Stuttgart; Georg Thieme Verlag 1988: 150-153
• 14 Vogl T J, Mack M G, Juergens M et al.. Skull base tumors: gadodiamide injection—enhanced MR imaging—drop-out effect in the early enhancement pattern of paragangliomas versus different tumors.  Radiology. 1993;  188 339-346
• 15 Sanna M, Piazza P, De Donato G, Menozzi R, Falcioni M. Combined endovascular-surgical management of the internal carotid artery in complex tympanojugular paragangliomas.  Skull Base. 2009;  19 26-42
• 16 Murphy T P, Brackmann D E. Effects of preoperative embolization on glomus jugulare tumors.  Laryngoscope. 1989;  99 1244-1247
• 17 Tran Ba Huy P, Kania R, Duet M, Dessard-Diana B, Mazeron J-J, Benhamed R. Evolving concepts in the management of jugular paraganglioma: a comparison of radiotherapy and surgery in 88 cases.  Skull Base. 2009;  19 83-91
• 18 Fisch U. Infratemporal fossa approach for extensive tumors of the temporal bone and base of the skull. In: Silverstein H, Norrel H Neurological Surgery of the Ear. Birmingham, AL; Aesculapius 1977: 34-53
• 19 Briner H R, Linder T E, Pauw B, Fisch U. Long-term results of surgery for temporal bone paragangliomas.  Laryngoscope. 1999;  109 577-583
• 20 Sanna M, Jain Y, De Donato G et al.. Management of jugular paragangliomas: the Gruppo Otologico Experience.  Otol Neurotol. 2004;  25 797-804
• 21 Selesnick S H, Abraham M T, Carew J F. Rerouting of the intratemporal facial nerve: an analysis of the literature.  Am J Otol. 1996;  17 793-805
• 22 Jackson C G, McGrew B M, Forest J A, Netterville J L, Hampf C F, Glasscock III M E. Lateral skull base surgery for glomus tumors: long-term control.  Otol Neurotol. 2001;  22 377-382
• 23 Gjuric M, Seidinger L, Wigand M E. Long-term results of surgery for temporal bone paraganglioma.  Skull Base Surg. 1996;  6 147-152
• 24 Mazzoni A. The petro-occipital trans-sigmoid approach for lesions of the jugular foramen.  Skull Base. 2009;  19 48-56
• 25 Makek M, Franklin D J, Zhao J C et al.. Neural infiltration of glomus temporale tumors.  Am J Otol. 1990;  11 1-5
• 26 George B, Lot G, Tran Ba Huy P. The juxtacondylar approach to the jugular foramen (without petrous drilling).  Surg Neurol. 1995;  44 279-284
• 27 Myssiorek D, Ferlito A, Silver C E et al.. Screening for familial paragangliomas.  Oral Oncol. 2007;  44 532-537